1: First Academic Department of Pneumonology, Interstitial Lung Diseases Unit, Hospital for Diseases of the Chest, "Sotiria", Medical School, National and Kapodistrian University of Athens, Athens, Greece

Abstract

Pulmonary fibrosis (PF) describes a condition in which the normal lung anatomy is replaced by a process of active remodeling, deposition of extracellular matrix and dramatic changes in the phenotype of both fibroblasts and alveolar epithelial cells, as a result of an abnormal wound healing process1-8. This condition can be idiopathic, as in idiopathic pulmonary fibrosis (IPF), or secondary to genetic disorders, lung parenchyma involvement in connective tissue disorders (CTDs), sarcoidosis9 or to exposure to environmental toxins or radiation. IPF is the most common idiopathic form of pulmonary fibrosis that affects approximately 5 million people worldwide, leading to death more than 100.000 patients each year, the same as breast cancer2. IPF, as well as other forms of progressive fibrotic lung disease, present with a median survival of 3-5 years from the time of diagnosis, evidence that makes them the non-cancer lung diseases with the gravest prognosis2,4-7.